Medicine of the Future in America

Category Archives: Hereditary hemorrhagic telangiectasia

Embolization of a duodenal arteriovenous malformation: CONCLUSIONS

When embolization fails to control GIH, angiography can guide surgical resection of the appropriate segment of bowel . However, the use of angiography has also been reported to misdirect surgical resection, leading to the removal of normal bowel . Catheter injection of methylene blue can be useful to stain involved regions of bowel for resection. Refractory cases in HHT and non-HHT patients often require eventual surgical management.

In the present patient, endoscopic treatment was not possible because the culprit lesion was not identified from within the lumen of the bowel. This is most likely due to the submucosal location of the lesion, anemia and the absence of active bleeding during investigation. In light of the nature of the lesion identified by angiography, medical therapy would not have been appropriate and did not offer a definitive solution. Furthermore, estrogen-progesterone was considered unacceptable to this patient due to the potential adverse effects. Surgery, although a potentially definitive therapy, is far more invasive than transcatheter embolization. Continue reading

Embolization of a duodenal arteriovenous malformation: DISCUSSION (Part 4)

small bowel AVMs treated by embolizationThere are no reports of small bowel AVMs treated by embolization in patients with HHT; however, there have been reports in non-HHT patients. In patients undergoing embolization for acute nonvariceal GIH, the bleeding lesion was devascularized by embolization in 39 of 40 patients . The most common source of bleeding in these patients were ulcers. In one non-HHT patient, a jejunal AVM was the source of bleeding and showed intermittent contrast medium extravasation during angiography . The jejunal artery was embolized with polyvinyl alcohol and the patient was asymptomatic for four months. However, when melena recurred, angiography was used to demonstrate revascularization of the previously embolized lesion. The microcatheter was then used for methylene blue marking to direct laproscopic resection of the involved jejunum and the patient was free of symptoms at 18 months. Continue reading

Embolization of a duodenal arteriovenous malformation: DISCUSSION (Part 3)

Medical therapy has a role in the management of GIH in some patients with HHT. Estrogen combined with progesterone has been shown to reduce the transfusion need in patients with bleeding malformations in the gastrointestinal tract, including six patients with HHT . However, estrogen can be poorly tolerated in men . Case reports of using aminocaproic acid have shown conflicting results in reducing the number of blood transfusions required in patients with HHT. Surgical management may be required in some patients with HHT to prevent ongoing GIH in refractory cases and in patients with larger AVMs thought to be less amenable to medical therapy or endoscopic management. Continue reading

Embolization of a duodenal arteriovenous malformation: DISCUSSION (Part 2)

duodenal AVM in a patient who may have had HHTThere is only one report describing a duodenal AVM in a patient who may have had HHT, based on a family history of a brother with recurrent GIH attributed to congenital telangiectasias. In non-HHT patients, angiodysplastic lesions are also more commonly reported than AVMs in the duodenum. In one review of 218 patients with bowel AVMs, only five patients were found to have lesions in the duodenum.

Diagnosis of gastrointestinal lesions in HHT can be made by endoscopy, angiography, technetium radionucleotide scan or laparotomy. Telangiectasias are usually easy to identify by endoscopy but they may be difficult to see in patients with profound anemia . Clinically important submucosal AVMs may not be recognized unless they are actively bleeding at the time of endoscopy. Furthermore, the yield may be influenced by the type of endoscopic device used. Traditional end-viewing esophagogastroduodenoscopes (at a length of 110 cm) allow visualization to the distal duodenum, to see beyond that, commercially available enteroscopes (200 cm to 250 cm) may be passed with or without an overtube . In certain cases a side-viewing duodenoscope would be optimal. Continue reading

Embolization of a duodenal arteriovenous malformation: DISCUSSION (Part 1)

HHT (also known as Osler-Weber-Rendu disease) is an autosomal dominant disorder characterized by vascular abnormalities, as recently reviewed. There are two types of vascular lesions in HHT. The first type is the mucocutaneous telangiectasia. Telangiectasias are small focal dilations of capillaries and postcapillary venules within the mucosa resulting in direct connections between arterioles and venules. When these lesions are found within the gastrointestinal tract they may be termed angiodysplasia. The second type of lesion is the visceral AVM. AVMs are direct connections between arteries and veins that lack intervening capillaries and are larger than telangiectasias or angiodysplasia. In patients with HHT, telangiectasias and/or AVMs may be found in the lungs, brain, skin, nose, liver and pancreas, as well as throughout the gastrointestinal tract. Continue reading

Embolization of a duodenal arteriovenous malformation: CASE PRESENTATION (Part 3)

superselective arteriography of the SMA No extravasation of contrast, representing active bleeding, was seen by angiography. The patient underwent superselective arteriography of the SMA to isolate the AVM (Figure 3). Platinum coil embolotherapy was performed, with total occlusion of the vessel feeding the malformation (Figure 4) and resolution of GIH. There were no complications from the procedure. In the following six months there was no history of recurrent GIH; however, the patient required transfusion with six units of RBCs due to ongoing epistaxis. Continue reading

Embolization of a duodenal arteriovenous malformation: CASE PRESENTATION (Part 2)

Ultrasound investigation of the abdomen identified a mildly inhomogeneous echo texture of the liver and a solid mass in mid pole of the left kidney consistent with a previously known renal oncocytoma. The gallbladder, common bile duct, pancreas, spleen and great vessels were normal. Mesenteric duplex ultrasound demonstrated markedly elevated flow within the celiac axis at slightly less than 4 L/min. Hepatic artery flow was abnormal, but difficult to accurately quantify. There was normal flow in the superior mesenteric artery (SMA) and in the right main and left portal veins. Continue reading

Embolization of a duodenal arteriovenous malformation: CASE PRESENTATION (Part 1)

hereditary hemorrhagic telangiectasiaA 68-year-old man with hereditary hemorrhagic telangiectasia (HHT) presented to hospital with recurrent gastrointestinal hemorrhage (GIH), manifesting as intermittent melena, for five years. Previous investigation by esophagogastroduo-denoscopy (EGD) revealed four telangiectasias in the stomach that were cauterized. Colonoscopy did not reveal the source of bleeding. The symptoms had progressed, and during the three months before admission to our hospital he had required transfusions of 27 units of packed red blood cells. Continue reading

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